Primary Amyloidosis Manifesting as Cholestatic Jaundice after Laparoscopic Cholecystectomy

Abstract

A 71-year-old female patient with cholelithiasis who had undergone laparoscopic cholecystectomy was admitted with obstructive jaundice (total bilirubin ~6 mg/dL) three months later. An ERCP was performed, in which a gallstone was found, followed by a sphincterotomy and cleansing of the bile duct. Due to deterioration of jaundice (>25 mg/dL), a new, unsuccessful ERCP and stent placement was carried out. Because of ongoing cardiac failure, she underwent an echocardiogram which revealed restrictive cardiomyopathy possibly due to amyloidosis. A liver biopsy was performed, which was positive for amyloid deposits in the liver, and the diagnosis was confirmed by the detection of monoclonal λ IgG protein in urine. The patient's jaundice gradually deteriorated and she died one week later from hepatic insufficiency.

Primary Hepatic Amyloidosis Presenting as Acute-on-Chronic Liver Failure

“…A recent study determined that only 0.44% of cases with liver failure were attributable to an infiltrating malignancy 3. Cases of acute liver failure have previously been reported in AL amyloidosis 4-10. These cases usually presented due to myeloma, which was absent in our patient.…”

“… 3 Cases of acute liver failure have previously been reported in AL amyloidosis. 4 - 10 These cases usually presented due to myeloma, which was absent in our patient. He did not have any chronic inflammatory condition, malignancy, or family history of amyloidosis.…”

“…ACLF has been defined by the Asian Pacific Association for the Study of the Liver (APASL) as an acute hepatic insult manifesting as jaundice and coagulopathy, complicated within 4 weeks by ascites and/or encephalopathy in a patient with previously diagnosed or undiagnosed chronic liver disease. 10 Among the infectious etiologies, reactivation of hepatitis B virus infection, either spontaneously or due to intensive chemotherapy or immunosuppressive therapy, as well as acute hepatitis E virus infection and hepatitis C virus infection have been reported. 11 Alcoholic hepatitis commonly causes decompensation of stable liver disease in various studies.…”

“…The clinical presentation of progressive liver failure with rapid deterioration and subsequent death in our case is therefore remarkable and is now being recognized as a distinct presentation of primary amyloidosis.material. Hepatocytes showed pressure 5 , 10 …”

“… 3 A diagnosis of AL amyloidosis (primary hepatic amyloidosis) with progressive liver failure is rarely seen in the absence of myeloma, with few prior cases of primary amyloidosis presenting as acute liver failure in the reviewed literature. 4 - 10 The diagnostic challenge in this case now serves as a differential in the etiological work-up for progressive liver failure.…”